cicatricisial alopecia
DESCRIPTION
LICHEN PLANOPILARIS CHRONIC CUTANEOUS LUPUS ERYTHEMATOUS CENTRAL CENTRIFUGAL CICATRICAL ALOPECIA PSEUDOPELADE OF BROCQ ALOPECIA MUCINOSIS KERATOSIS PILARIS SPINULOSA DECALVANSTRANSCRIPT
TUTORIAL PRESENTATION
CICATRICIAL ALOPECIA
Definition:Permanent area of hair loss associated with destruction of hair follicles
Pilosebaceous structures are replaced by fibrous tracts Classified as:
1. Primary 2. Secondary
Trauma Sclerosing disorders Granulomatous disorders Infections Neoplastic
3. Developmental/Hereditary PCA more common than SCA (4:1)
ANOTHER CLASSIFICATION PROPOSED TO FACILITATE DETERMINATION OF MOST SUITABLE SURGICAL CORRECTIVE THERAPIES FOR CA :
stable - Traumatic Aplasia cutis CCCA
unstable – Lymphocytic Neutrophilic Mixed Infections Congenital Neoplastic
PATHOGENESIS HFSCs destruction theories Impairment of self maintenance of HFSCs Alteration of lipid metabolism Neurogenic inflammation theory Environment factors Genetic factors
PPAR DEFICIENCY CAUSES LOSS OF PEROXISOME BIOGENESIS, DEREGULATES LIPID METABOLISM, AND PRODUCES PROINFLAMMATORY LIPIDS THAT TRIGGER INFLAMMATORY RESPONSE THAT IN TURN CAUSES TISSUE DAMAGE AND PERMANENT HAIR LOSS
NORMALIN CICATRICIAL
ALOPECIA
APPROACH TO THE PATIENT
History Onset Presence of- pruritus, irritation, pain, erythema and/or
drainage from the scalp Evaluate for autoimmune disease, systemic illness,
infections, neoplasms, associated inflammatory skin disease and radiation treatment or burns
Drug intake Clinical Findings
loss of follicular ostia erythema, scaling, pustules, scalp bogginess and
compound follicles (polytrichia) Biopsy
Non-scarring hair
Scarring hair loss
Incidence More common Less common
Erythema/scaling/pustules
-/+ +
Atrophy absent present
Loss of follicular openings
absent present
Tufted hair absent present
Course regrowth is quite common
no regrowth
Prognosis generally favourable
generally unfavourable
PRIMARY CICATRICAL ALOPECIA
Diagnosis in which lymphocytes predominates includes:
LICHEN PLANOPILARISCHRONIC CUTANEOUS LUPUS ERYTHEMATOUSCENTRAL CENTRIFUGAL CICATRICAL ALOPECIAPSEUDOPELADE OF BROCQALOPECIA MUCINOSISKERATOSIS PILARIS SPINULOSA DECALVANS
GRAHAM-LITTLE PICCARDI LASSUEUR SYNDROMEAFFECTS WOMEN BETWEEN 30-70 YRSSYNDROME CHARACTERIZED BY:PROGRESSIVE CICATRICIAL ALOPECIA OF SCALP,NON SCARRING ALOPECIA OF AXILLA AND PUBIC AREA AND KERATOSIS PILARIS
Classic lichen planus40% of pt have skin manifestations
C/Fs: violaceous papules,erythema scaling
Papules replaced by follicular plugs
Plugs shed and finally atrophic,smooth,scarred area remains
Pt commonly presents with pseudopelade like patches
Frontal Fibrosing AlopeciaResembles AGA with frontal recession
C/Fs:perifollicular erythema and hyperkeratoses at marginal hairline
Slow progressive disease
Typically occurs in Post-menopausal women
Central centrifugal cicatricial alopeciahot comb alopecia, follicular degeneration syndrome, pseudopelade in African Americans central elliptical pseudopelade
Premature disintegration of inner root sheath epithelium occurs
Begins as single focus over vertex of scalp and then spread centrifugally
Pseudopelade
Idiopathic,chronic,slowly progressive
Patchy cicatricial alopecia that occur without any evidence of inflammation
footprints in the snow
Chronic Cutaneous Lupus Erythematosus
30% have skin manifestationErythema, scaling and pigmentary changes are more pronounced Follicular plugging and adherent scale may be present. The “carpet tack” sign may be elicited with retraction of scale revealing keratotic spikes that correspond to follicular openings on undersurface
Keratosis follicularis spinulosa decalvans
X-Linked recessive SSAT gene defectErythema, plugging of eyebrow follicles follicular hyperkeratosis & prominent cuticles Ocular signs include blepharitis, ectropion, corneal dystrophy and photophobiaFocal PPK may be present
Diagnosis in which neutrophils predominates includes:
FOLLICULITIS DECALVANSDISSECTING CELLULITIS OF SCALP
Folliculitis decalvans
-Recurrent crops of follicular pustules that result in permanent epilation
-Staph aureus may be grown from pustules
-Pustular folliculitis followed by rounded patches of alopecia develop surrounded by crusting and few follicular pustules.
-Successive crops of pustules appear and are followed by progressive destruction of affected follicles
-Tufted folliculitis variant of folliculitis decalvans where circumscribed areas of scalp inflammation heal with scarring characterized by tufts of up to 15 hairs emerging from single orifice
Perifolliculitis of scalp, deep and superficial abscesses in dermis, sinus tract formation and extensive scarring
Aetiology:staphylococci, streptococci and Pseudomonas may be cultured from various lesions
C/Fs:Painful, firm, skin-coloured nodules develop near vertex
Confluent nodules form tubular ridges with an irregular cerebriform pattern
Progressive scarring and permanent alopecia occur
Chronic condition with frequent acute exacerbations.
Dissecting cellulitis of the scalp
Perifolliculitis capitis abscedens et suffodiens
Diagnoses in which a mix of cell types predominate are as follows:
ACNE KELOIDALISACNE NECROTICAEROSIVE PUSTULAR DERMATOSES OF SCALP
Acne keloidalis
It occurs in males after puberty between the ages of 14 -25 yrs
C/Fs:Pts present with pustules, alopecia and hypertrophic scarring on posterior neck
Friction from the collar is often incriminated
Process begins with penetration of cut hair into
the skin as in pseudofolliculitis
Acne necrotica
More frequent in men than in women
30- 50 yrs
C/Fs:red itchy acneiform papules arise spontaneously on the front and sides of scalp
papules are usually centered around pilosebaceous unit
Often umbilicated and rapidly transformed by necrosis into an adherent haemorrhagic crust which separates after 3 or 4 weeks to leave a permanent varioliform scar
Particularly affects the elderly Precipitating factors:local trauma
sundamage,surgery, cryosurgery skin grafting and radiation therapy
Initially, a small area of scalp becomes red, crusted and irritable
crusting and superficial pustulation overlie a moist, eroded surface
As condition extends areas of activity coexist with areas of scarring.
Squamous carcinoma has developed in scars
Erosive pustular dermatosis of the scalp
SECONDARY CICATRICIAL ALOPECIA
GRANULOMATOUS DISORDERS
C/F:The oval atrophic plaques on the shins but may be seen on other parts of body including scalp.
The patches are glazed, yellowish often with conspicuous telangiectasia
Scarring may be dense. Clinical features in scalp vary
from large plaques of cicatricial alopecia to multiple small areas of scarring
Cutaneous sarcoidosis may produce plaques or nodules on scalp
Necrobiosis lipoidica, granuloma annulare and sarcoidosis
Circumscribed scleroderma and linear morphoea
rare in the scalp
’en coup de sabre’ morphoea – is more common
Cicatricial pemphigoidWomen > men
disease predominantly affects ocular and/or genital mucous membrane
skin is involved in 40–50%
scalp involved in 10% of cases
SCLEROSING DISORDERS
TRAUMATIC
Common in Afro-Caribbean hair styles
Due to sustained pull on hair roots
Folliculitis , hair casts reduction in hair density
with vellus hairs and sometimes broken hairs
Hair loss begins in temporal regions and in front of and above the ears but may involve other parts of scalp
CHILD: Scalp electrodes or infusion or forceps delivery or uterine rings in neonate can result in trauma.
ADULT:Brain surgery, gynaecological surgery in Trendelenburg position
Traction alopecia Medical trauma
TRICHOTILLOMANIA
Behavioural disorder characterized by compulsive hair pulling Trichoteiromania : Compulsive hair rubbing Trichotemnomania : Compulsive hair cutting Hair is plucked most frequently from one frontoparietal region Patch of hair loss bizarre or angular pattern in which hairs
are twisted and broken at various distances from clinically normal scalp
H/P: Numerous empty canals , clefts in hair matrix, intraepithelial and perifollicular haemorrhages and intrafollicular pigment casts
Some follicles are severely damaged Follicular epithelium is separated from connective tissue sheath Trichomalacia - Injured follicles may form only soft, twisted hair
TRACTION ALOPECIA. TRACTION FOLLICULITISIS COMMONLY ASSOCIATED
Syphilitic alopecia.
The scalp has moth-eaten appearance, eyebrow hair is absent there is rash on the cheek
Trichotillomania. Hairs are thin
and of different lengths
TRACTION ALOPECIADERMATOPHYTE:INFECTION
DIAGNOSIS
Dermoscopy/Trichoscopy - first-line, noninvasive method
Absence of follicular ostia in 100% cases even if it is not evident clinically
FFA :loss of orifices, perifollicular scale and feeble perifollicular erythema
Folliculitis decalvans :existence of micropustules and/or hair tufting with >=6 hairs
DLE: follicular red dots LPP: hair tufting, violaceous-blue interfollicular area,
corresponding to pigment incontinence Lipedematous alopecia: linear area of telangiectasia within
scalp creases, possibly caused by compression of the superficial blood capillaries
Scalp sarcoidosis: orange spots seen(round, well-formed granulomas in superficial dermis)
Traction alopecia: Hair casts
Reflectance confocal microscopy Microscopic imaging of superficial layers of skin
down to superficial reticular dermis with resolution at cellular level close to conventional histopathology
May also help in choosing most appropriate biopsy site for more informative histology
HistopathologyDirect immunofluorescenceMicroarray analysis
LUPUS ERYTHEMATOUS: H/P:VACUOLAR INTERFACE ALTERATION OF FOLLICULAR EPITHELIUM, SCATTERING OF DYSKERATOTIC KERATINOCYTES, VARIABLY DENSE PERIADNEXAL, PERIFOLLICULAR (UPPER PORTION), PERIVASCULARAND INTERSTITIAL LYMPHOCYTIC INFILTRATE WITH DERMAL MUCIN, ATROPHY OF SEBACEOUS GLANDS AND FOLLICULAR PLUGGING. EPIDERMIS MAY BE ATROPHIED WITH VACUOLAR INTERFACE CHANGES. CONCENTRIC LAMELLAR FIBROSIS AROUND THE FOLLICLE IN END STAGES
follicular plugging, superficial,deep perivascular and periappendageal lymphocytic infiltrate
There is linear staining of deposits of complement (C3), IgM and IgG on the basement membrane in more than 80% of cases of LE
LICHEN PLANUS: ACTIVE : FOLLICULAR LYMPHOCYTIC INTERFACE DERMATITIS WITH DENSE BAND LIKE LYMPHOCYTES AROUND UPPER FOLLICLE & INFUNDIBULUM OBSCURING DEJ, INFUNDIBULAR HYPERKERATOSIS AND HYPERGRANULOSIS, CYTOID BODIES SCATTERED ALONG THE BMZ, ABSENT OR ATROPHIC SEBACEOUS GLANDS WITH OR WITHOUT PIGMENTARY INCONTINENCE. END –STAGE : LONGITUDINAL TRACTS OF FIBROSIS,LAMELLAR FIBROSIS,EPIDERMAL ATROPHYDIF:‘SHAGGY’ OR ‘PATCHY’ DEPOSITION OF FIBRINOGEN AND CLUMPED IGM OR LESS COMMONLY IGA AND C3 DEPOSITS ARE SEEN ALONG FOLLICULAR BMZ
Variably dense perifollicular lymphocytic infiltrate in early stage, followed by eccentric atrophy of follicle infundibular epithelium, concentric lamellar fibrosis around upper follicle and loss of sebaceous gland in later stage
Elastin stains reveal dense elastic tissue cuffing a broad fibrotic follicular tract in advanced disease
thinned out epidermis with total loss of hair follicles, replaced by collagen
Pseudopelade of Brocq
Central centrifugal cicatricial alopecia :Earliest feature is premature disintegration of IRS resulting in outward migration of hair shaft through ORS at level of isthmus. Lamellar fibroplasias and lymphocytic inflammation surround the follicle at this level, resulting in follicular destruction and fibrous tract formation.
Alopecia mucinosa :Mucinous degeneration of ORS and sebaceous glands. A perifollicular lymphocytic infiltrate often with eosinophils and histocytes
Keratosis follicularis spinulosa decalvans :Compact hyperkeratosis, hypergranulosis of upper follicular epithelium with superficial intrafollicular and peri-follicular edema in early stage whereas in advanced stage there is concentric perifollicular, horizontal adventitial lamellar fibrosis and scarred follicular tracts
Folliculitis decalvans: Acneiform dilatation with perifollicular neutrophilic inflammation- later mixed inflammatory infiltrate of neutrophils, lymphocytes, plasma cells. Follicular rupture,foreign-body giant cell granuloma formation around exposed hair shaft fragments. In burnt outstage, follicular and adventitial fibrosis is seen
Dissecting cellulitis of scalp: Infundibular acneiform distention with intrafollicular and perifollicular neutrophilic infiltration, abscess formation,sinus tracts
Acne keloidalis : perifollicular and intrafollicular lymphoplasmacytic infiltrate at the level of sebaceous glands -complete follicular destruction occurs with loss of sebaceous glands and dermal fibrosis
Acne necrotica:dense perivascular and perifollicular lymphocytic infiltrate with prominent sub-epidermal edema. Necrosis of individual keratinocyte is seen initially and is followed by confluent necrosis of the central follicle and interfollicular epidermis
TREATMENT
Aim of treatment currently focuses reduction of symptoms and to reduce or stop progression of disease.
LMPCA with immunosuppression NMPCA with antimicrobials or dapsone
First line Class I or II potent topical
corticosteroids I/L steroid inj (10 mg/ml max 2 ml,
every 4-6 weeks) Results are assessed till 8 weeks, if
no response shift to next levelSecond line Antimalarials (HCQ 200-400 mg/day,
clinical effect in 4-8 weeks, continued till 3-6 mths)
Oral corticosteroids (1 mg/kg, for initial actively progressing disease, tapered over 8 weeks)
Retinoids (acitretin and Isotretinoin 10-40 mg/day)
Third line Thalidomide, topical
immunomodulators , oral vit E, gold, dapsone,MMF,methotrexate, azathioprine,clofazamine,systemic or intralesional INFα2, monoclonal anti-CD4 antibodies, topical 5-FU, topical tazarotene imiquimod.
First line Potent topical corticosteriods I/L Triamcinolone acetonide
Second line Oral corticosteroids Oral cyclosporine(4-5 mg/kg for 4-
6 mths) Topical cyclosporine (oily solution,
applied twice daily for initial 3 mths and once daily for further 3 mths)
Oral tetracycline
Third line Retinoids ,Antimalarials,MMF(500
mg twice daily) Others:
Thalidomide,griseofulvin,low molecular weight heparin (s/c injections 3 mg once weekly), exicmer laser
Newer therapies PPARγ agonist like
thiozolidinediones
DLE LPP
Intralesional triamcinolone acetonide
Finasteride (2.5 mg OD) Oral corticosteroids Antimalarials Topical corticosteroids with topical
minoxidil Oral retinoids
GLP Topical and intralesional
corticosteroids Oral ciclosporin Systemic corticosteroids Topical tacrolimus
PPB Potent topical corticosteroids(±)
First line Potent topical corticosteriodsSecond line I/L triamcinolone Minocycline DapsoneThird line Systemic steroids,isotretinoin,
antimalarials,PUVA,interferon α-2b+Interferon γ,superficial X-rays
KFSD Oral antibiotics Dapsone Oral retinoids Laser epilation
FFA Alopecia mucinosa
First line Oral±topical antibioticsSecond line Oral rifampicin (300 mg BD)
+oral clindamycin (300 mg BD) Rifampicin+ (doxycycline/ciprofl
oxacin/clarithromycin) Oral rifampicin+topical
antibiotics
Third line Oral fusidic acid Oral zinc Dapsone Oral cyclosporine Excision,laser,radiotherapy,i/m
Human immunoglobulin
First line Oral isotretinoin Oral isotretinoin+i/l
triamcinolone acetonideSecond line Oral antibiotics+topical
antibiotics/topical retinoids Aspiration and i/l triamcinolone
acetonideThird line Low dose corticosteroids Colchicine Dapsone Excision and skin grafting Lasers and radiotherapy
FD Dissecting cellulitis of scalp
First line Potent topical steroid Oral antibiotics+topical
steroids/intralesional triamcinolone
Second line Surgical excision CO2 laser Diode laser hair epilationThird line Radiotherapy Isotretinoin
Oral antibiotics Oral isotretinoin I/L triamcinolone
Erosive pustular dermatosis of scalp
Topical corticosteroids Topical
immunomodulators Calcipotriol cream Oral Isotretinoin
Acne keloidalis nuchae Acne necrotica varioliformis
Surgical treatment of scarring alopecia includes : hair transplantation scalp reduction or alopecia reduction surgeries tissue expansion flap surgeries
CONCLUSION
CA is ‘trichology emergency’ situation, in which lack of prompt and early treatment will lead to the inevitable loss of hair follicles along with permanent scarring.
Newer pathogenesis has given the platform for development of emerging treatment modalities.
But still great deal of research is required in this field, may be developing viable stem cell therapies or bioengineered human hair follicles are the answers to it in future
THANK YOU